Hereditary deficiencies in complement C5 are associated with intensified neurodegenerative responses that implicate new roles for the C-system in neuronal and astrocytic functions.

abstract：BACKGROUND:Alzheimer's disease (AD) is the most common neurodegenerative disorder. Depositions of amyloid β peptide (Aβ) and tau protein are among the major pathological hallmarks of AD. Aβ and tau burden follows predictable spatial patterns during the progression of AD. Nevertheless, it remains obscure why certain bra...

journal_title：Neurobiology of disease

authors： Mendonça CF,Kuras M,Nogueira FCS,Plá I,Hortobágyi T,Csiba L,Palkovits M,Renner É,Döme P,Marko-Varga G,Domont GB,Rezeli M

更新日期：2019-10-01 00:00:00

abstract：:Dystonia and levodopa-induced dyskinesia (LID) are both hyperkinetic movement disorders. Dystonia arises most often spontaneously, although it may be seen after stroke, injury, or as a result of genetic causes. LID is associated with Parkinson's disease (PD), emerging as a consequence of chronic therapy with levodopa,...

journal_title：Neurobiology of disease

authors： Calabresi P,Standaert DG

更新日期：2019-12-01 00:00:00

abstract：:Parkinson's Disease (PD) and Multiple System Atrophy (MSA) are neurodegenerative diseases characterized neuropathologically by alpha-synuclein accumulation in brain cells. This accumulation is hypothesized to contribute to constitutive neuroinflammation, and to participate in the neurodegeneration. Cytokines, which ar...

journal_title：Neurobiology of disease

authors： Rydbirk R,Elfving B,Andersen MD,Langbøl MA,Folke J,Winge K,Pakkenberg B,Brudek T,Aznar S

更新日期：2017-10-01 00:00:00

abstract：:Previous studies have indicated that administration of glial cell line-derived neurotrophic factor (GDNF) counteracts neuronal death after stroke. However, in these studies damage was evaluated at most a few days after the insult. Here, we have explored the long-term consequences of two routes of GDNF delivery to the ...

journal_title：Neurobiology of disease

authors： Arvidsson A,Kirik D,Lundberg C,Mandel RJ,Andsberg G,Kokaia Z,Lindvall O

更新日期：2003-12-01 00:00:00

abstract：:Vascular cognitive impairment is a major cause of dementia caused by chronic hypoxia, producing progressive damage to white matter (WM) secondary to blood-brain barrier (BBB) opening and vascular dysfunction. Tight junction proteins (TJPs), which maintain BBB integrity, are lost in acute ischemia. Although angiogenesi...

journal_title：Neurobiology of disease

authors： Yang Y,Kimura-Ohba S,Thompson JF,Salayandia VM,Cossé M,Raz L,Jalal FY,Rosenberg GA

更新日期：2018-06-01 00:00:00

abstract：:Dravet syndrome is a severe infantile-onset epileptic encephalopathy which begins with febrile seizures and is caused by heterozygous loss-of-function mutations of the voltage-gated sodium channel gene SCN1A. We designed a CRISPR-based gene therapy for Scn1a-haplodeficient mice using multiple guide RNAs (gRNAs) in the...

journal_title：Neurobiology of disease

authors： Yamagata T,Raveau M,Kobayashi K,Miyamoto H,Tatsukawa T,Ogiwara I,Itohara S,Hensch TK,Yamakawa K

更新日期：2020-07-01 00:00:00

abstract：:Symptoms in Parkinson's Disease (PD) have been linked to oscillatory activity within the basal ganglia. In humans, such activity has been detected mainly in the local field potentials (LFPs) recorded from electrode contacts used for deep brain stimulation. Although most studies have focused on activity within the subt...

journal_title：Neurobiology of disease

authors： Tsiokos C,Hu X,Pouratian N

更新日期：2013-06-01 00:00:00

abstract：:The disruption of the blood-spinal cord barrier (BSCB) by matrix metalloprotease (MMP) activation is a detrimental event that leads to blood cell infiltration, inflammation, and apoptosis, thereby contributing to permanent neurological disability after spinal cord injury (SCI). However, the molecular mechanisms underl...

journal_title：Neurobiology of disease

authors： Lee JY,Na WH,Choi HY,Lee KH,Ju BG,Yune TY

更新日期：2016-11-01 00:00:00

abstract：:We have used a quantitative in situ hybridization method with human ribonucleotide probes to examine the regional and cellular distribution of N-methyl-D-aspartate receptor (NMDAR) subunit mRNAs in the human cerebellum. Purkinje cells showed very dense labeling for NMDAR1 mRNA, dense labeling for NMDAR2A mRNA, and mod...

journal_title：Neurobiology of disease

authors： Scherzer CR,Landwehrmeyer GB,Kerner JA,Standaert DG,Hollingsworth ZR,Daggett LP,Veliçelebi G,Penney JB Jr,Young AB

更新日期：1997-01-01 00:00:00

abstract：:Exercise has beneficial effects on brain function, including the promotion of plasticity and the enhancement of learning and memory performance. Previously we found that exercise increases the expression of certain neurotrophic factors including brain derived neurotrophic factor in the rat hippocampus. To further expl...

journal_title：Neurobiology of disease

authors： Tong L,Shen H,Perreau VM,Balazs R,Cotman CW

更新日期：2001-12-01 00:00:00

abstract：:Long-term consequences of status epilepticus (SE) occur in a significant proportion of those who survive the acute episode. We developed an in vivo model of acute focal neocortical SE (FSE) to study long-term effects on local cortical structure and function and potential strategies to mitigate adverse consequences of ...

journal_title：Neurobiology of disease

authors： Perez-Ramirez MB,Gu F,Prince DA

更新日期：2020-08-01 00:00:00

abstract：OBJECTIVE:In Parkinson's disease, chronic striatal dopamine depletion results in over-activity and under-activity of the indirect and direct striatal output pathways respectively. In this study, we investigated changes in the function of glutamatergic cortico-striatal synapses that contribute to abnormalities in striat...

journal_title：Neurobiology of disease

authors： Warre R,Thiele S,Talwar S,Kamal M,Johnston TH,Wang S,Lam D,Lo C,Khademullah CS,Perera G,Reyes G,Sun XS,Brotchie JM,Nash JE

更新日期：2011-03-01 00:00:00

abstract：:Spinocerebellar ataxia type 3 (SCA3) or Machado-Joseph disease (MJD) belongs to a group of autosomal dominant neurodegenerative diseases, which are caused by the expansion of a polyglutamine repeat in the affected protein, in this case ataxin-3. Ataxin-3 is mainly localized in the cytoplasm; however, one hallmark of S...

journal_title：Neurobiology of disease

authors： Antony PM,Mäntele S,Mollenkopf P,Boy J,Kehlenbach RH,Riess O,Schmidt T

更新日期：2009-11-01 00:00:00

abstract：:Cerebral ischemia activates endogenous neurogenesis in the subventricular zone (SVZ) and the dentate gyrus. Consecutively, SVZ-derived neural precursors migrate towards ischemic lesions. However, functional relevance of activated neurogenesis is limited by poor survival of new-born precursors. We therefore employed th...

journal_title：Neurobiology of disease

authors： Doeppner TR,Dietz GP,El Aanbouri M,Gerber J,Witte OW,Bähr M,Weise J

更新日期：2009-04-01 00:00:00

abstract：:Endocannabinoids function as neurotransmitters and neuromodulators in the central nervous system via specific receptors and apparently have a neuroprotective role. We assumed that the endocannabinoid system could be involved in the pathogenesis of hepatic encephalopathy (HE), a neuropsychiatric syndrome due to liver d...

journal_title：Neurobiology of disease

authors： Avraham Y,Israeli E,Gabbay E,Okun A,Zolotarev O,Silberman I,Ganzburg V,Dagon Y,Magen I,Vorobia L,Pappo O,Mechoulam R,Ilan Y,Berry EM

更新日期：2006-01-01 00:00:00

abstract：:Traumatic brain injury (TBI) leads to acute necrosis at the site of injury followed by a sequence of secondary events lasting from hours to weeks and often years. Targeting mitochondrial impairment following TBI has shown improvements in brain mitochondrial bioenergetics and neuronal function. Recently formoterol, a h...

journal_title：Neurobiology of disease

authors： Vekaria HJ,Hubbard WB,Scholpa NE,Spry ML,Gooch JL,Prince SJ,Schnellmann RG,Sullivan PG

更新日期：2020-07-01 00:00:00

abstract：:GPNMB is a glycoprotein observed upon tissue damage and inflammation and is associated with astrocytes, microglia, and macrophages. Gene variations in GPNMB are linked with Parkinson's disease (PD) risk, and changes in protein levels of GPNMB have been found in lysosomal storage disorders, including Gaucher's disease ...

journal_title：Neurobiology of disease

authors： Moloney EB,Moskites A,Ferrari EJ,Isacson O,Hallett PJ

更新日期：2018-12-01 00:00:00

abstract：:Tissue transglutaminase (tTG) is an indicator of acute cell death in vitro. An increase in tTG protein level is found in postmortem Alzheimer's disease (AD) brains as well as in Huntington's disease. No study revealed tTG in vivo so far. We investigated the concentrations of tTG in the cerebrospinal fluid (CSF) obtain...

journal_title：Neurobiology of disease

authors： Bonelli RM,Aschoff A,Niederwieser G,Heuberger C,Jirikowski G

更新日期：2002-10-01 00:00:00

abstract：:Toll-like receptors (TLRs) are mediators of the innate immune response to exogenous pathogens. They have also been implicated in sterile inflammation associated with systemic injury and non-infectious diseases via binding of endogenous ligands, possibly released by damaged cells. Emerging evidence indicates that some ...

journal_title：Neurobiology of disease

authors： David BT,Ratnayake A,Amarante MA,Reddy NP,Dong W,Sampath S,Heary RF,Elkabes S

更新日期：2013-06-01 00:00:00

abstract：:The impact of Abeta deposition upon cholinergic intrinsic cortical and striatal, as well as basal forebrain long projection neuronal systems was qualitatively and quantitatively evaluated in young (2-6 months) and middle-aged (10-16 months) APPswe/PS1DeltaE9 transgenic (tg) mice. Cholinergic neuritic swellings occurre...

journal_title：Neurobiology of disease

authors： Perez SE,Dar S,Ikonomovic MD,DeKosky ST,Mufson EJ

更新日期：2007-10-01 00:00:00

abstract：:Extracellular nucleotides exert a variety of biological actions through different subtypes of P2 receptors. Here we characterized in the human neuroblastoma SH-SY5Y cells the simultaneous presence of various P2 receptors, belonging to the P2X ionotropic and P2Y metabotropic families. Western blot analysis detected the...

journal_title：Neurobiology of disease

authors： Cavaliere F,Nestola V,Amadio S,D'Ambrosi N,Angelini DF,Sancesario G,Bernardi G,Volonté C

更新日期：2005-02-01 00:00:00

abstract：:Proteolytic cleavage of the amyloid precursor protein (APP) by the two proteases α- and β-secretases controls the generation of the amyloid β peptide (Aβ), a key player in Alzheimer's disease pathogenesis. The α-secretase ADAM10 and the β-secretase BACE1 have opposite effects on Aβ generation and are assumed to compet...

journal_title：Neurobiology of disease

authors： Colombo A,Wang H,Kuhn PH,Page R,Kremmer E,Dempsey PJ,Crawford HC,Lichtenthaler SF

更新日期：2013-01-01 00:00:00

abstract：:Huntington's disease (HD) is one of the few neurodegenerative diseases with a known genetic cause, knowledge that has enabled the creation of animal models using genetic manipulations that aim to recapitulate HD pathology. The study of behavioral and neuropathological phenotypes of these HD models, however, has been p...

journal_title：Neurobiology of disease

authors： Menalled L,El-Khodor BF,Patry M,Suárez-Fariñas M,Orenstein SJ,Zahasky B,Leahy C,Wheeler V,Yang XW,MacDonald M,Morton AJ,Bates G,Leeds J,Park L,Howland D,Signer E,Tobin A,Brunner D

更新日期：2009-09-01 00:00:00

abstract：:Amyotrophic lateral sclerosis (ALS) is characterized by a progressive loss of large motor neurons in the brain and spinal cord. Amyloid precursor protein (APP), the transmembrane precursor of beta-amyloid (A beta), accumulates in the anterior horn motor neurons of ALS patients with mild lesions. APP undergoes an alter...

journal_title：Neurobiology of disease

authors： Calingasan NY,Chen J,Kiaei M,Beal MF

更新日期：2005-06-01 00:00:00

abstract：:Patients with Krabbe disease, a genetic demyelinating syndrome caused by deficiency of galactosyl-ceramidase and the resulting accumulation of galactosyl-sphingolipids, develop signs of a dying-back axonopathy compounded by a deficiency of large-caliber axons. Here, we show that axonal caliber in Twitcher mice, an ani...

journal_title：Neurobiology of disease

authors： Cantuti-Castelvetri L,Zhu H,Givogri MI,Chidavaenzi RL,Lopez-Rosas A,Bongarzone ER

更新日期：2012-05-01 00:00:00

abstract：:The amyloid precursor protein (APP) is a type I transmembrane protein translocated to neuronal terminals, whose function is still unknown. The C-terminus of APP mediates its interaction with cellular adaptor and signaling proteins, some of which signal to the stress-activated protein kinase (SAPK) pathway. Here we sho...

journal_title：Neurobiology of disease

authors： Galvan V,Banwait S,Spilman P,Gorostiza OF,Peel A,Ataie M,Crippen D,Huang W,Sidhu G,Ichijo H,Bredesen DE

更新日期：2007-10-01 00:00:00

abstract：:Human genetics provides unbiased insights into the causes of human disease, which can be used to create a foundation for effective ways to more accurately diagnose patients, stratify patients for more successful clinical trials, discover and develop new therapies, and ultimately help patients choose the safest and mos...

journal_title：Neurobiology of disease

authors： Finkbeiner S

更新日期：2020-12-01 00:00:00

abstract：:Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder caused by an excessive expansion of a CAG trinucleotide repeat in the gene encoding the protein huntingtin, resulting in an elongated stretch of glutamines near the N-terminus of the protein. Here we report the derivation of a collection of ...

journal_title：Neurobiology of disease

authors： Castiglioni V,Onorati M,Rochon C,Cattaneo E

更新日期：2012-04-01 00:00:00

abstract：:We investigated two measures of neural integrity, T1-weighted volumetric measures and diffusion tensor imaging (DTI), and explored their combined potential to differentiate pre-diagnosis Huntington's disease (pre-HD) individuals from healthy controls. We applied quadratic discriminant analysis (QDA) to discriminate pr...

journal_title：Neurobiology of disease

authors： Georgiou-Karistianis N,Gray MA,Domínguez D JF,Dymowski AR,Bohanna I,Johnston LA,Churchyard A,Chua P,Stout JC,Egan GF

更新日期：2013-03-01 00:00:00

abstract：:As researchers across the globe have focused their attention on understanding SARS-CoV-2, the picture that is emerging is that of a virus that has serious effects on the vasculature in multiple organ systems including the cerebral vasculature. Observed effects on the central nervous system include neurological symptom...

journal_title：Neurobiology of disease

authors： Buzhdygan TP,DeOre BJ,Baldwin-Leclair A,Bullock TA,McGary HM,Khan JA,Razmpour R,Hale JF,Galie PA,Potula R,Andrews AM,Ramirez SH

更新日期：2020-12-01 00:00:00